Acute exacerbation of idiopathic pulmonary fibrosis: international survey and call for harmonisation.

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focussed international guidelines for the management of AE-IPF exist. The aim of this international survey was to assess the global variability in prevention, diagnostic and treatment strategies for AE-IPF.Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel.509 pulmonologists from 66 countries responded. Significant geographical variability in approaches to manage AE-IPF was found. Common preventive measures included antifibrotic drugs and vaccination. Diagnostic differences were most pronounced regarding use of Krebs von den Lungen-6 and viral testing, while high-resolution computed tomography, brain natriuretic peptide and D-dimer are generally applied. High-dose steroids are widely administered (94%); the use of other immunosuppressant and treatment strategies is highly variable. Very few (4%) responders never use immunosuppression. Antifibrotic treatments are initiated during AE-IPF by 67%. Invasive ventilation or extracorporeal membrane oxygenation are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (64%).Approaches to the prevention, diagnosis and treatment of AE-IPF vary worldwide. Global trials and guidelines to improve the prognosis of AE-IPF are needed.

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Conflict of interest: M. Kreuter reports grants and personal fees from Roche, Galapagos and Boehringer, outside the submitted work. Conflict of interest: M. Polke has nothing to disclose. Conflict of interest: S.L.F. Walsh reports personal fees for consultancy from Sanofi-Aventis, Galapagos and OSIC, personal fees for advisory board work from Roche, grants and personal fees for steering committee work from Boehringer Ingelheim, personal fees for lectures from Bracco, outside the submitted work. Conflict of interest: J. Krisam has nothing to disclose. Conflict of interest: H.R. Collard reports personal fees from Bayer, Boehringer Ingelheim, Bristol-Myers Squibb, Global Blood Therapeutics, ImmuneWorks, Navitor, Parexel, Prometic, Toray, Unity, Patara, Veracyte, Roche/Genentech, aTyr, Advance Medical and MedImmune, grants from Pulmonary Fibrosis Foundation, grants and personal fees from Three Lakes Partners, outside the submitted work. Conflict of interest: N. Chaudhuri reports grants from Boehringer Ingelheim and Roche, educational support from Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: S. Avdeev has nothing to disclose. Conflict of interest: J. Behr has nothing to disclose. Conflict of interest: G. Calligaro has nothing to disclose. Conflict of interest: T. Corte reports grants, personal fees for lectures and advisory board work, and travel support from Boehringer, grants and personal fees for lectures and advisory board work from Roche, grants from Galapagos, Actelion, Bayer and Sanofi, personal fees for advisory board work from AstraZeneca, outside the submitted work. Conflict of interest: K. Flaherty reports grants and personal fees from Boehringer Ingelheim and Roche/Genentech, personal fees from Bellerophon, Respivant, Veracyte, Sanofi-Genzyme, Blade Therapeutics and Celgene, outside the submitted work. Conflict of interest: M Funke-Chambour has nothing to disclose. Conflict of interest: M. Kolb reports grants and personal fees for consultancy and lectures from Roche and Boehringer Ingelheim, grants and personal fees for consultancy from GSK, Gilead and Prometic, grants from Actelion, Respivert, Alkermes and Pharmaxis, personal fees for consultancy from Genoa, Indalo and Third Pole, outside the submitted work. Conflict of interest: Y. Kondoh reports advisory board fees and lecture fees from Asahi Kasei Pharma Corp. and Boehringer Ingelheim Co., Ltd, advisory board fees from Janssen Pharmaceutical K.K., lecture fees from Eisai Inc., Kyorin Pharmaceutical Co., Ltd, Mitsubishi Tanabe Pharma, Novartis Pharma K.K. and Shionogi and Co., Ltd, outside the present work. Conflict of interest: T.M. Maher has, via his institution, received industry-academic funding from GlaxoSmithKline R&D and UCB, and has received consultancy or speaker fees from Apellis, AstraZeneca, Bayer, Blade Therapeutics, Boehringer Ingelheim, Bristol-Myers Squibb, Galapagos, GlaxoSmithKline R& D, Indalo, Novartis, Pliant, ProMetic, Respivnat, Roche, Samumed and UCB. Conflict of interest: M. Molina Molina reports grants and personal fees from Roche, Boehringer Ingelheim and Esteve-Teijin, grants from GSK and AstraZeneca, personal fees from Pfizer and Chiesi, outside the submitted work. Conflict of interest: A. Morais has nothing to disclose. Conflict of interest: C.C. Moor has nothing to disclose. Conflict of interest: J. Morisset reports personal fees from Hoffmann La Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: C. Pereira has nothing to disclose. Conflict of interest: S. Quadrelli has nothing to disclose. Conflict of interest: M. Selman has nothing to disclose. Conflict of interest: A. Tzouvelekis has nothing to disclose. Conflict of interest: C. Valenzuela reports personal fees for advisory board work and lectures from Roche and Boehringer Ingelheim, personal fees for advisory board work from Galapagos, outside the submitted work. Conflict of interest: C. Vancheri reports grants and personal fees from Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: V. Vicens-Zygmunt has nothing to disclose. Conflict of interest: J. Wälscher has nothing to disclose. Conflict of interest: W. Wuyts has nothing to disclose. Conflict of interest: M. Wijsenbeek reports grants and fees paid to institution from Boehringer Ingelheim and Hoffman la Roche, fees paid to institution from Galapagos and Novartis, outside the submitted work. Conflict of interest: V. Cottin reports personal fees for advisory board work and lectures, and non-financial (travel) support from Actelion, grants, personal fees for consultancy and lectures, and non-financial (travel) support from Boehringer Ingelheim and Roche, personal fees for advisory board and data monitoring committee work from Bayer/MSD and Galapagos, personal fees for adjudication committee work from Gilead, personal fees for advisory board work and lectures from Novartis, personal fees for lectures from Sanofi, personal fees for data monitoring and steering committee work from Promedior, personal fees for data monitoring committee work from Celgene and Galecto, outside the submitted work. Conflict of interest: E. Bendstrup reports grants and personal fees from Boehringer Ingelheim and Roche, outside the submitted work.